Microbiological and Molecular Study of Paranasal Sinus Infections of Children with Malignancy and Unknown Origin Fever in Markazi Province, Iran.

Background: Children with malignancies are vulnerable to various infections, including sinus infections. Sinusitis is primarily caused by bacterial infections, followed by fungal infections. Due to this, evaluating the occurrence, diversity, and antibiotic susceptibility patterns of bacterial species that cause paranasal sinus infections in children with malignancy and unexplained fever is important. Objective: To investigate the bacterial species accountable for sinusitis in children with malignancy and unexplained fever, and determine their susceptibility to antibiotics. Methods: The study involved collecting 90 sinus samples from children aged 5 to 15 years with malignancy in Arak City, Iran. The isolates were identified using a combination of phenotypic, biochemical, and molecular techniques, including specific polymerase chain reaction and 16S ribosomal RNA gene sequencing. Drug susceptibility testing was performed following the Clinical & Laboratory Standards Institute 2021 guidelines. Results: A total of 36 isolates (40%) were obtained, including 4 isolates of Nocardia (11.12%), 4 isolates of Escherichia coli (11.12%), 3 isolates of Klebsiella pneumoniae (8.33%), 5 isolates of Pseudomonas aeruginosa (13.88%), 3 isolates of Acinetobacter baumannii (8.33%), 4 isolates of Staphylococcus aureus (11.12%), 3 isolates of Staphylococcus epidermidis (8.33%), 5 isolates of Streptococcus agalactiae (13.88%), 2 isolates of Streptococcus pneumoniae (5.55%), and 3 isolates of Enterococcus faecium (8.33%). The isolates showed the most sensitivity to imipenem and trimethoprim-sulfamethoxazole and the least sensitivity to erythromycin and tetracycline. Conclusions: The findings of the study indicate that sinusitis can contribute to fever of unknown origin in patients with cancer. Therefore, it is recommended to use a combination of molecular and phenotypic methods for accurate identification of isolates. This approach can provide more reliable and precise results, leading to better diagnosis and treatment of sinusitis infections in children with malignancy.

A Rare Case of Klippel Trenaunay Syndrome with Von Willebrand Factor Deficiency and Multiple Accessory Spleens: A Case Report and Brief Literature Review.

Klippel Trenaunay Syndrome (KTS) is an uncommon inherited syndrome identified by venous varicosities and capillary abnormalities. von Willebrand Disease is the most common inherited hemorrhage disturbance in humans, leading to insufficiency in von Willebrand Factor, which is a complex multimeric protein with two functions: it forms a bridge between the platelets and injured vascular areas and it attaches factor VIII and stabilizes it. We present a 13-year-old son with a typical clinical manifestation of KTS, including "port-wine stains" as capillary malformation, venous malformation, and hypertrophy of the left lower extremity, who also suffers from von Willebrand Disease type 3. He has been suffering from these two rare conditions since birth. The occurrence of KTS with von Willebrand Factor deficiency in a patient has so far not been reported, which may propose a mutation in the putative common regulatory gene that caused this uncommon phenotype.

Correlation of Magnesium Level with Cardiac and Hepatic Hemosiderosis Measured by T2*MRI Technique and Age in Patients with Thalassemia Major.

Background: Given the association of hypomagnesemia with cardiac arrhythmia, the aim of this study was to investigate the relationship between serum magnesium levels with age and T2* magnetic resonance imaging (MRI) findings of the heart and liver in patients with thalassemia major (TM). Materials and Methods: In a descriptive cross-sectional study, a total of 62 patients with ß-thalassemia major aged 11-48 years were selected at the Amir-Kabir Hospital, Arak, Iran. Serum magnesium, ferritin, and iron levels of patients were measured, and the rate of cardiac and hepatic hemosiderosis of patients was extracted according to the routine T2*MRI method. Results: The mean age of the patients at diagnosis was 32.6 years. The comparison of TM patients with and without hepatic/cardiac hemosiderosis demonstrated that mean levels of serum ferritin, serum iron, and age were significantly higher in TM patients with cardiac hemosiderosis than in hepatic/cardiac non-hemosiderosis (P < 0.05); however, there was no significant difference in mean levels of serum magnesium in TM patients with and without hepatic/cardiac hemosiderosis (P = 0.279). Interestingly, the correlation of age with serum magnesium levels in TM patients revealed a statistically significant and moderate inverse correlation (r = -0.56, P = 0.013). Conclusion: Hypomagnesemia may occur in a time-dependent manner. It is recommended that, in addition to cardiac and hepatic T2*MRI, serum magnesium levels be measured by using magnesium replacement if necessary.

The Role of Hematological Parameters in Predicting the Death of Hospitalized Patients with COVID-19.

Background: The role of the hematologic indicators in the identification of severe or critical patients requires further investigation. In this study, we focused on predicting Covid-19 patients at risk of progression using blood parameters. Materials and Methods: We performed a retrospective study including 444 patients with confirmed Covid-19. Hematological parameters were evaluated. The logistic regression analysis was performed with step-wise method with dependent variables such as intensive care units admission, partial pressure of oxygen saturation, and mortality. Also, independent variables such as hematological parameters, age and sex to assess variables that are likely to predict patients at risk of progression. Results: Patients in intensive care units had significantly higher mean absolute neutrophil count than outpatients (P < 0.001). There was a statistically significant difference in the mean absolute lymphocyte count between dead and survived patients (P = 0.015). Multivariate analysis confirmed the positive association of the white blood cells (P < 0.001), absolute neutrophil count (P < 0.004), red cell distribution width (P < 0.001), and lactate dehydrogenase (P = 0.007) to be positively associated with the admission of Covid-19 patients in the intensive care units and the absolute monocyte count (P = 0.012, Odds ratios = 0.100, CI95% = 0.066-0.605) to be negatively associated with mortality. Conclusion: Based on the results of our study, it is recommended to use hematological data to make clinical decisions and evaluate the patient's prognosis.

Kidney Failure in Children with Wilms Tumor: A Study Based on Urine Analysis and Ultrasound.

Background: Renal insufficiency is one of the inevitable complications in patients with Wilms tumor (WT). The purpose of this study was to assess the renal function in children with WT at baseline and every 3 months to 2 years. Materials and Methods: In a descriptive-analytical study from 2018 to 2020, 48 children with WT were included in the study. Urine creatinine (UCr), serum calcium (SCr), blood pressure (BP), estimated glomerular filtration rate (eGFR), and urinary protein (UPro) were evaluated at baseline and every 3 months during the study. Spot UCa/UCr and spot UPro/UCr ratio were calculated. Kidney ultrasonography was used in all patients. Independent Sample t-test and Chi-square tests were utilized to compare age and sex, respectively. Results: The mean age of patients at follow-up was 7.3 years. There was no significant difference in mean UCr, SCr, eGFR, 24-h UPro, UCa/UCr ratio, and spot UPro/UCr ratio at baseline and end of study (P baseline> 0.05, P end of study> 0.05). Analysis of kidney size showed a statistical association with tumor stage (P < 0.05). Comparison of the kidney size in patients showed that there is a statistically significant difference (P < 0.0001) at baseline and end of the study. Conclusion: This study showed that as WT progressed, the size of the kidneys increases without any renal insufficiency. Therefore, it seems that urinalysis of patients with WT along with sonography is necessary to determine renal insufficiency and the use of ultrasound alone to determine kidney insufficiency is not recommended.

Renal Function in Patients with Thalassemia Major Receiving Exjade® Dispersible Tablets and a New Film-coated Tablet Formulation of Deferasirox (Nanojade®).

Background: In patients with ß-thalassemia major (TM), one of the long-term complications of regular blood transfusion is renal dysfunction. The purpose of the current study was to evaluate the renal function in TM patients receiving Exjade® dispersible tablets and a new film-coated tablet formulation of deferasirox (Nanojade®). Materials and Methods: In this descriptive cross-sectional study, a total of 80 TM patients aged 11-48-year-old entered the study. Patients received 20-30 mg/kg/d (single dose) Exjade® (Exjade group, n = 40) and Nanojade® (Nanojade group, n = 40) orally. To evaluated renal function, serum creatinine (SCr), estimated glomerular filtration rate (eGFR), blood urea nitrogen (BUN), 24-h urine protein (UPro), UCa/UCr, spot UPro/UCr ratio, and serum ferritin were calculated at baseline and every 3 months to 9 months. Results: There was no significant difference in SCr, BUN, eGFR, 24-h UPro, UPro/UCr ratio, UCa/UCr ratio, and serum ferritin between groups at baseline and end of study (P baseline> 0.05, P end of study> 0.05). There was no significant difference in proteinuria between groups at baseline and end of study (P baseline> 0.05, P end of study> 0.05). Conclusions: The proportion of SCr, BUN, eGFR, 24-h UPro, UPro/UCr ratio, and UCa/UCr ratio was not significantly different in TM patients treated with Nanojade® compared to patients' received Exjade®. Nanojade® had similar effects to Exjade®, and therefore, the use of Nanojade® is safe in TM patients and does not seem to be associated with increased renal failure, proteinuria, and hypercalciuria.

A Retrospective Cytogenetic Abnormality in Pediatric Acute Lymphoblastic Leukemia: Report of 11 Years.

Background: Acute lymphoid leukemia (ALL) is the largest subset of hematologic malignancies, accounting for approximately 70%-80% of childhood leukemia, and is most common at age 4 years. The aim of this study was to define the frequency of chromosomal abnormalities in pediatric ALL. Materials and Methods: In this 11-year retrospective study, we investigated 99 patients which referred to our department due to ALL from 2010 to 2020. The age group of the patients ranged from 6 months to 14 years with a mean of 6.71 ± 4.09 years. Clinical and diagnostic findings were extracted from patients' medical records. Results: We showed cytogenetic abnormalities of 99 pediatric ALL patients, including 78 pre-B-ALL, 9 common B-ALL, and 12 T-ALL cases. The 5-year overall survival rate (OSR) and event-free survival (EFS) of all cytogenetic abnormalities (n = 99) were 48% and 43%, respectively. There was a significant relationship between the two cytogenetic abnormalities, hypodiploidy and t(9;22), with death (P < 0.05). On comparing the subjects with normal cytogenetics to the other cytogenetic abnormalities, EFS was significantly low for hypodiploidy (P = 0.0163, hazard ratio = 0.5308) and t(9;22) (P = 0.0131, hazard ratio = 0.4908), while other cytogenetic abnormalities did not have a statistically significant difference in EFS. Conclusions: Our results emphasized the importance of the cytogenetic findings in evaluating the survival outcomes, which allows identifying a variety of OSR and EFS, because some of the cytogenetic abnormalities may interfere with the death and prognosis.

Evaluation of Blood Pressure in Pediatric Survivors of Acute Lymphoblastic Leukemia and Healthy Children; A Case-control Study.

Background: The exact prevalence of hypertension in children surviving acute lymphoblastic leukemia (ALL) has not been fully estimated. The aim of this study was to investigate the prevalence of arterial hypertension (AH) and to determine the risk factors for the development of AH in children surviving ALL with current treatments. Materials and Methods: A total of 150 patients (84 males, 66 females, with an age range of 1-16 years) were included in the study. Demographic and clinical information of patients were initially recorded. Hypertension is defined as average systolic blood pressure (BP) and/or diastolic BP that is greater than the 95th percentile for gender, age, and height. Results: The mean age at the assessment of BP was 11.3 and 9.8 years in the ALL and control group, respectively. A total of 20.6% of survivors of ALL and 10% of controls had high BP. Most patients in both groups had normal BP (65.3% patients in ALL group and 75.4% subjects in the control group). The number of patients with hypertension was significantly higher in ALL patients as compared with the control group (P = 0.026). Conclusion: The prevalence of AH in children surviving ALL is higher than in children in the general population, which emphasizes the need for regular monitoring of BP in children surviving ALL and intervention in the lifestyle of this population. Careful follow-up of BP status is warranted for long-term survivors of childhood cancer.

Comparison of the effect of ferrous sulfate and ferrous gluconate on prophylaxis of iron deficiency in toddlers 6-24 months old: A randomized clinical trial.

BACKGROUND: Iron deficiency anemia (IDA) is one of the most common anemias, especially in children 4-23 months. Therefore, prophylaxis is necessary to improve iron status as well as reduce IDA in Toddlers. The aim of this study was to compare the efficacy of daily supplementation with ferrous gluconate (FG) and ferrous sulfate (FS) on iron status in toddlers. MATERIALS AND METHODS: A total of 120 healthy toddlers were divided randomly into 2 groups at the Amir-Kabir Hospital, Arak, Iran and received FS and FG from March 2020 to December 2020. Iron status was evaluated at baseline and after 6 months of supplementation. The statistical significance of the differences in iron status between FS and FG groups was calculated using Student's t-test and the Pearson' s Chi-square test for qualitative variables. SPSS software (version 16, Chicago, IL, USA) was used for statistical analysis. RESULTS: Comparison of iron status of FS and FG groups toddlers at baseline and after 6 months of supplementation showed that there was a significant difference in hemoglobin (Hb) (10.46 vs. 12.45, P = 0.001) and ferritin level (28.08 vs. 59.63, P = 0.001). CONCLUSIONS: Although prophylaxis with FG led to a higher Hb and ferritin levels, our study recommended that both FG and FS supplements were effective for prophylactic use in the prevention of IDA. However, FG was more effective than FS because FG group that received FG supplementation indicated a higher Hb and ferritin levels in comparison to the FS group that received FS supplementation.

Evaluation of the Relationship Among Pulmonary Artery Pressure with Serum Ferritin Level and CMR T2* in Thalassemia Major.

BACKGROUND: Pulmonary hypertension is one of the most common cardiac complications among patients with ß-thalassemia major (ß-TM). The aim of this study is to investigate the correlation of pulmonary artery pressure (PAP), serum ferritin level, and cardiac T2* MRI in patients with ß-TM. METHOD: This cross-sectional study was performed on 50 patients older than 7 years old. Echocardiography, electrocardiography, and cardiac T2* MRI were performed on all patients and their serum ferritin levels were measured. Based on the echocardiographic the patients were divided into two groups of PAP>30 and <30 mmHg. RESULTS: 40% had PAP higher than 30 mmHg, 32% had abnormal T2* MRI, and 36% had serum ferritin levels higher than 1500ng/dl. there were significant negative correlations between CMRI *T2 and PAP (-0.36) and the P-pulmonary (-0.29). the serum ferritin level was positively correlated with PAP (0.44) and the P pulmonary in ECG (0.30). in the patients with PAP>30, the means of age, serum ferritin level, and P-pulmonary were significantly higher than patients with PAP<30 (P=0.001). In the patients with PAP>30mmHg, CMRI *T2 had higher accuracy (80%), sensitivity (65%), and specificity (90%) compared to the measurement of serum ferritin levels. CONCLUSION: The PAP was positively correlated with serum ferritin levels and negatively with T2MRI. The accuracy of 80% for T2MRI as a method to diagnose or even anticipate PH among patients with TM suggested that this method is a useful and accurate method of PH diagnosis and can be used as an alternative among clinicians.

Aspergillosis of central nervous system in patients with leukemia and stem cell transplantation: a systematic review of case reports.

BACKGROUND: Aspergillosis of Central Nervous System (CNS) is a highly lethal infection in patients with leukemia and Stem Cell Transplantation (SCT). METHODS: Case reports of CNS aspergillosis in patients with leukemia and SCT published between 1990 and August 2020 were gathered using a structured search through PubMed/Medline. RESULTS: Sixty-seven cases were identified over the searches of the PubMed bibliographic database and then, 59 cases were included in the final analysis. Europe had the largest share of cases at 57.6% (34 reports), followed by Americas and Asia. Affected patients were predominantly males (58.6%) and the mean age of the patients was 36.1 years, while 62.7% of the patients were under the age of 50 years. The most common leukemia types include Acute Lymphoblastic Leukemia (ALL), Chronic Lymphocytic Leukemia (CLL), and Acute Myeloid Leukemia (AML) at 43.4%, 27.4%, and 23.5%, respectively. Furthermore, stem cell transplantation was reported in 11 cases. The overall mortality was 33%; however, the attributable mortality rate of CNS aspergillosis was 24.5%. Altered mental status, hemiparesis, cranial nerve palsies, and seizures were the clearest manifestations of infection and lung involvement reported in 57% of the patients. Histopathologic examination led to the diagnosis of infection in 57% of the patients followed by culture (23.7%), galactomannan assay (8.5%), and molecular method (3.3%). Amphotericin B and voriconazole were the most frequently used drugs for infection treatment. Good results were not obtained in one-third of the patients treated by voriconazole. Finally, neurosurgical intervention was used for 23 patients (39%). CONCLUSION: CNS aspergillosis is a rapidly progressive infection in leukemic patients. Thus, these patients should be followed up more carefully. Furthermore, management of induction chemotherapy, use of different diagnostic methods, and use of appropriate antifungal can lead to infection control.